Mauriac Syndrome in a Patient of Type 1 Diabetes Mellitus

Abstract

Mauriac syndrome is a metabolic complication of poor glycaemic control in Type 1 DM patients, unique to children, characterised by growth failure, short stature, delayed puberty, Cushingoid features, and marked hepatomegaly from massive deposition of glycogen. It is probably due to a combination of factors including inadequate glucose uptake and utilisation in the tissues, decreased insulin-like growth factor-1 and growth hormone levels, impaired bioactivity of these hormones, a circulating hormone inhibitor, resistant or defective hormone receptors, insulin deficiency, and poor glycaemic control. This syndrome is rarely seen nowadays because of better treatment modalities.